Inborn errors of amino acid metabolism: Information from Answers.com

Inborn errors of amino acid metabolism
Classification and external resources
The general structure of an α-amino acid, with the amino group on the left and the carboxyl group on the right.
ICD-10	E 70.-E 72.
ICD-9	270
MeSH	[1]

Inborn errors of amino acid metabolism are metabolic disorders which impair the synthesis and degradation of amino acids.

Types include:

Amino acid transport disorders

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Inborn error of amino acid metabolism (E70-72, 270)

Lysine/straight chain	Glutaric acidemia type 1 · type 2 · Hyperlysinemia · Pipecolic acidemia · Saccharopinuria

Leucine	Maple syrup urine disease · Isovaleric acidemia · 3-Methylcrotonyl-CoA carboxylase deficiency · 3-hydroxy-3-methylglutaryl-CoA lyase deficiency

Tryptophan	Hypertryptophanemia

Histidine	Carnosinemia · Histidinemia · Urocanic aciduria

Proline	Hyperprolinemia · Prolidase deficiency

Glutamate/glutamine	SSADHD

Valine	Maple syrup urine disease · Hypervalinemia · Isobutyryl-CoA dehydrogenase deficiency

Isoleucine	Maple syrup urine disease · Beta-ketothiolase deficiency · 2-Methylbutyryl-CoA dehydrogenase deficiency

Methionine	Hypermethioninemia · Homocystinuria · Cystathioninuria

General BC/OA	Propionic acidemia · Methylmalonic acidemia · Methylmalonyl-CoA mutase deficiency


Urea cycle/Hyperammonemia (arginine, aspartate)	N-Acetylglutamate synthase deficiency · Carbamoyl phosphate synthetase I deficiency · Ornithine transcarbamylase deficiency/translocase deficiency · Citrullinemia · Argininosuccinic aciduria · Argininemia

Other

see also Amino acid metabolism enzymes, intermediates
see also Urea cycle enzymes, intermediates

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